Hello strangers…it’s been a while… …but put away your hankies, and dry your eyes, for the Knackered Girls are BACK and more knackered than ever! Yes folks, we have been resting since we completed our Way of the Roses coast … Continue reading
We just wanted to share with you an update that Evan’s parents shared on his Just Giving page.
It’s a little longer & more serious than our usual blog posts but please do read. It’s very important.
1 year on and we have some answers:-
We met with Evan’s consultant on Friday at Alder Hey following news that they had found a genetic mutation responsible for his HLH.
Following his death last year our consultant continued to fight for ‘full’ genetic testing to be done on his DNA. Evan had been ‘screened’ for genetic mutations 4 times throughout his illness and on each occasion the results had been negative and therefore full genetic testing was deemed not necessary. On the 15th of August she finally won her fight to get Evan full genetic testing despite all his screening results being negative as her belief was that his disease was a genetic form. So full genetic testing began at Great Ormond Street hospital in London. On the 22nd April this year (his 5th birthday) they found and reported a diagnosis of XLP type 2. (X-linked lymphoproliferative disease or X-linked familial hemophagocytic lymphohistiocytosis) It took them 8 months to find this, genetic testing is very complex, difficult, expensive and only by means of ellimination can they find a mutation if one exists. Hense the fight our consultant had to get this done for Evan.
I will try to simplify and explain what this means. XLP is a disorder of the immune system. It means that Evan produced abnormally large numbers of the natural killer cells that our immune systems produce to fight infection and virus’s. This leads to a reaction called HLH that causes fever, destroys blood producing cells in the bone marrow, damages the liver and attacks other organs and tissues within the body. It truly is a horrible disease. Undetected it will kill in a matter of weeks. HLH is what they knew Evan was suffering from but they didn’t know why. To try to hold the disease they use Chemotherapy, steroids and immune supressants to kill off some of the killer cells doing all the damage until they can get the patient stable enough to transplant as this is their only hope of survival. Normally when we produce killer cells they only live for a few days in our body and die off, However the mutation that Evan had protects the killer cells so that they do not die off and continue to multiply and destroy. Unfortunately over the past 2 years very few HLH patients have successfully come through transplant, something that is not yet understood, for some reason when transplanting the effect it has on the liver and gut in HLH patients seems increase in severity. They transplant many leukemia patients without issue and have not yet found out the differerence the transplant procedure has on the HLH patients, only more research, questioning, learning, trying will lead to answers.
Don’t forget to donate to Three Knackered Girls at: justgiving.com/threeknackeredgirls
Hopefully now you can see why your donations count & make such a big difference & can help us keep moving forward with research.
Today, 29th May, it is exactly a year since Evan left us.
Our thoughts are with those who were closest to him – those who loved him & who he loved back.
Evan went through so much in his four years with us, including chemotherapy & a bone marrow transplant. He was a remarkable little boy who gave so much & fought so hard.
We would like to ask you to donate £1 today in his memory.
You can do this online at: justgiving.com/threeknackeredgirls
Or by texting EVAN70 £1 to 70070
You can spread the word by using hashtag: #1pound4evan
Here at Knackered Girls HQ (which is mainly a spiritual plane of higher silliness as we’re all in different counties) we have started putting together a prize. We’re looking to crowd source a bunch of suitable goodies & have all ready been pledged a couple of bottles of champagne which means we’re off to an EXCELLENT start!
If you are in the Yorkshire area or can post to the Yorkshire area then & think you might have a little something lurking in the back of your cupboard, if you are a dab hand at baking biscuits or chocolatiering (that’s a real thing…honest) & you’d like to contribute then we’d love to hear from you!
Drop us an email at: firstname.lastname@example.org or by using the contact form below to find out how to get your little something to us!
Please help spread the word. Remember that this is all to raise vital funds for research into the disease which took little Evan from us & will help us get closer to a cure.
Your pot of jam or pack of biccies could help us save a life one day & that’s pretty darn cool, if you ask me! Thanks for reading!
As you may have seen we finally have some beautiful pictures of Evan on the site, with thanks to his parents – Joe & Russ – for allowing us to put these up here.
We hope this will help those who didn’t know him to see what a charming, cheeky & gorgeous little boy he was. In all of the pictures we have here his personality shines through.
All the more reason for us to get our thinking caps on & start planning the extra challenges we will be doing in between all the cycling. We’ve had suggestions of beermat building challenges, fancy dress, treasure hunts but we just don’t know what to choose & so we would like a little help from our firends (that’s you!).
What would YOU like to see Three Knackered Girls & their crew undertake?
Could it be like Top Gear, except with bikes rather than cars & total glamour (AHEM) instead of greying middle aged men?
Could it be like some survivor show where we compete to be Queen Tribeswoman & forage for food in the wilderness? (Please say no!)
Could it be that we have a Woman vs Food type challenge every evening? Imagine the bloatedness the next day…!
What could it be folks?
Comment below with your suggestion & share amongst your friends. There might even be a prize for the best suggestion…Ooooooh!
~ Clo x
G’day to you.
We will be blogging shortly.
God help you all.
For now, why not DONATE in memory of Evan.
Thanks for stopping by!